Association of central pontine myelinolysis and Marchiafave-Bignami disease

Combined pathologic features of central pontine myelinolysis and Marchiafava-Bignami disease occurred in a 53-year-old man with chronic alcoholism. This association is rare despite the basic similarities of these two disorders. The combination of both conditions in the same patient suggests a common pathogenetic mechanism.     

Myelopathy presenting as an intrinsic spinal cord tumor

Two patients are presented who became progressively quadriplegic with myelographic appearances suggesting intrinsic spinal cord tumors. In both cases, laminectomy and biopsy of the spinal cord disclosed non-neoplastic conditions (neuromyelitis optica and acute necrotic myelopathy) confirmed at autopsy. Since surgery is not helpful in such cases, the problems of differentiation between such lesions and intraspinal tumors are reviewed.     

Spinal adrenal cortical adenoma with oncocytic features: report of the first intramedullary case and review of the literature

Ectopic adrenal cortical neoplasms are extremely rare, and only a few have involved the CNS. We report the first case of an intramedullary oncocytic adrenal cortical neoplasm of the spinal cord with immunohistochemical (IMHC) confirmation. A 27-year-old man presented with progressive lower extremity weakness, spastic paraparesis, decreased reflexes, and hypoesthesia below T10. A spinal myelogram showed cauda equina blockade and obliteration of sacral nerve roots. This prompted emergent surgical intervention. A well-circumscribed, approximately 3 x 2 cm, light brown to tan, intramedullary tumor was identified at the level of the conus medullaris. Histologically, the tumor showed sheets and nests of plump, cytologically bland polygonal cells with abundant eosinophilic cytoplasm. A single mitosis, but no necrosis, was identified. By IMHC, the cells were positive for inhibin, melan-A, and synaptophysin, and negative for GFAP, EMA, cytokeratins, S-100, HMB-45, and chromogranin. Electron microscopy study performed from paraffin-embedded tissues demonstrated abundant mitochondria, and lipid vacuoles. This case confirms the occurrence of adrenal cortical neoplasms in the CNS and is the first report of an intradural, intramedullary adrenal cortical adenoma of the spinal cord, and the first to occur in a male. This tumor should be considered in the differential diagnosis of tumors of the CNS.     

Chondrosarcoma of the arytenoids- a rare laryngeal malignancy

Even though laryngeal malignancies are the most frequent primary malignancies of the upper aero digestive tract except for oral cavity cancers, laryngeal chondrosarcomas are rare tumors, constituting less than 1% of all laryngeal tumors. We present a rare case of chondrosarcoma arising from the right arytenoid cartilage with sub glottic extension. The mode of presentation and management of the case is presented along with a review of the literature.     

Intrasellar primitive neuroectodermal tumor (PNET) in familial retinoblastoma: a variant of "trilateral retinoblastoma"

An infant with bilateral familial retinoblastomas was found at age 6 months to have a large mass within the region of the sella turcica. The histology, immunoreactivity and ultrastructure of the tumor showed primitive neuroectodermal cells. A small number of cells showed positive immunostaining to retinal S antigen. This represents a rare form of so-called trilateral retinoblastoma. In most cases of trilateral retinoblastoma, the intracranial non-metastatic tumor is within the pineal gland. The occurrence of such a tumor in the region of sella turcica is very unusual and its possible significance is discussed in regard to our current understanding of the susceptibility to carcinogenesis conferred by inheritance of the mutant retinoblastoma gene, Rb-1.     

Malignant transformation of benign cerebellar astrocytoma

A patient who had a well-differentiated cerebellar astrocytoma resected at 4 years of age, had glioblastoma multiforme of the cerebellum after a symptom-free interval of 28 years. Late malignant transformation of a cerebellar astrocytoma of childhood is rare and previous cases are reviewed. Cerebellar astrocytomas are typically among the most benign of primary brain malignancies with excellent long-term survival rates, sometimes with incomplete resection. This patient indicates that follow-up needs to be long-term because biologic behavior cannot be predicted fully in all cases.     

Brain changes associated with thromboxane receptor antagonist SQ 29,548 treatment in a mouse model

The purpose of this study was to characterize behavioral and physiological effects of a selective thromboxane (TP) receptor antagonist, SQ 29,548, in the C57Bl/6 mouse model. At 6 months of age, male mice were given either sham or drug i.p. injections for 3 days at a dose of 2 mg/kg each day. On the day after the final injection, mice were subjected to behavioral testing before brain collection. Left hemisphere hippocampi were collected from all mice for protein analysis via Western blot. Right brain hemispheres were fixed and embedded in gelatin and then serially sectioned. The sections were immunostained with anti‐c‐Fos antibodies. Prostaglandin analysis was performed from remaining homogenized brain samples, minus the hippocampi. Injection of SQ 29,548 decreased selective brain prostaglandin levels compared with sham controls. This correlated with robust increases in limbic‐region c‐Fos immunoreactivity in the SQ 29,548‐injected mice. However, drug‐treated mice demonstrated no significant changes in relevant hippocampal protein levels compared with sham treatments, as determined from Western blots. Surprisingly, injection of SQ 29,548 caused mixed changes in parameters of depression and anxiety‐like behavior in the mice. In conclusion, the results indicate that administration of peripheral TP receptor antagonists alters brain levels of prostanoids and influences neuronal activity, with only minimal alterations of behavior. Whether the drug affects neurons directly or through a secondary pathway involving endothelium or other tissues remains unclear. © 2015 Wiley Periodicals, Inc.     

Anaphylaxis in the United States: an investigation into its epidemiology

BACKGROUND: Anaphylaxis is a severe, life-threatening allergic reaction that affects both children and adults in the United States. However, data regarding the incidence and prevalence of anaphylaxis and the number of deaths caused by it are limited. OBJECTIVE: To provide a better understanding of the magnitude of the problem of anaphylaxis in the United States. METHODS: A thorough review of the current medical literature was conducted to obtain prevalence estimates on each of the 4 major subtypes of anaphylaxis (food, drugs, latex, and insect stings). We calculated an overall estimate of the risk of anaphylaxis by using only estimates that are specifically derived from epidemiologic studies measuring anaphylaxis in the general population. RESULTS: Known rates or cases of anaphylaxis were 0.0004% for food, 0.7% to 10% for penicillin, 0. 22% to 1% for radiocontrast media, and 0.5% to 5% after insect stings. There were 220 cases after latex exposure. Considering the 1999 US population of 272 million, the population at risk for anaphylaxis from food is 1099, from penicillin is 1.9 million to 27. 2 million, from radiocontrast media is 22 000 to 100 000, from latex is 220, and from insect stings is 1.36 million to 13.6 million. These calculations yield a total of 3.29 million to 40.9 million individuals at risk of anaphylaxis. CONCLUSION: The occurrence of anaphylaxis in the US is not as rare as is generally believed. On the basis of our figures, the problem of anaphylaxis may, in fact, affect 1.21% to 15.04% of the US population.